The clinical and histopathological findings in a case of inflammatory linear verrucose epidermal nevus coexisting with lichen amyloidosus are presented. A 33-year-old woman had had linear verrucose lesions which were histopathologically compatible with ILVEN, and with which no amyloid deposits were associated, on her left lower leg for 30 years. She had noticed keratotic papules around the verrucose lesions on the left leg for the last 3 years. Histopathology of these papules revealed amyloid deposits in the upper dermis under the hyperkeratotic and acanthotic epidermis. Topical application of DMSO liniment with dexamethasone successfully treated the ILVEN. This remission of both lesions and itching also alleviated the keratotic papules on the left leg.